Feer Disease

FEER DISEASE (Feer) is described under various names: disease Selter-Swift-Feer's, 'vegetative neurosis of young children,' trophodermoneurosis, paralysis of the vegetative system, dermatopolyneuritis, acrodynia infantilis, pink disease (red disease), erythroedema and others. The etiology of F. d. is unknown, probably of infectious origin; it is characterized by disturbance of the general state (depressed mood, restless sleep, poor appetite), millet-like rash with subsequent peeling, mainly on palms and soles, sweating, cyanosis or redness of peripheral parts (nose, hands, feet), muscle hypotonia, weakening of motor ability, and finally increased pulse rate and elevated blood pressure. History. The first to describe this disease in 1903 (8 cases) was Selter from Sodingen in Germany, who called it trophodermoneurosis, but his report did not attract attention. In 1914, Swift from Australia reported on 14 cases of the same disease, which he named erythroedema. Subsequently (1914-1921), reports of a number of cases appeared in America. In 1923, Feer, who was not aware of all these works, described a 'new' disease (6 cases), which he called vegetative neurosis of young children. Zechlin by means of a questionnaire to children's clinics in Europe established that by 1929 in Germany 111 cases were registered, in Switzerland-36 (almost all from Feer's clinic), in France about 30, in Holland-17; individual cases were described in Austria, Czechoslovakia, Poland and other countries. In the USSR, F. d. was first described in 1931 by E. Ya. Gindes from Baku, and then in 1934 by V. I. Molchanov (Moscow). Epidemiology. F. d. is a disease of young children. The vast majority of patients are aged 6 months-4 years; much less common at ages 3-6 months or over 7 years. Cases among adults have been described in Holland and Belgium, but Feer doubts their belonging to this disease. Patients are almost equally divided between boys and girls. The disease occurs more often in winter and autumn months (from December to May). In some countries, the distribution of the disease is observed in foci and islands; such focal distribution was proven by Feer for Switzerland, by Zechlin for Germany. Etiology and pathogenesis. The available information is completely insufficient and contradictory. Some tend to consider F. d. or acrodynia as a clinical syndrome that can be caused by various harmful factors: auto-intoxication from the intestine, encephalitic virus ('acrodynic syndrome' of Dutch authors). As proof of the closeness and even identity of Feer's disease with lethargic encephalitis, Dutch authors (Wyggelendam and Kuiper) cite cases of family disease they described, in which adults showed symptoms of encephalitis. Kühl on the basis of clinical observations and results of patho-anatomical research expressed the assumption that the basis of F. d. lies in dysfunction of the cortical and medullary substance of the adrenal glands. With the normal course of post-uterine development of the adrenal glands, as is known, a deep restructuring of the gland occurs at exactly the time when F. d. is most often observed, i.e. at the age of 1 year to 4 years. With deviation from the normal course of development, premature and enhanced development of the medullary substance occurs, resulting in hypofunction of the cortical and hyperfunction of the medullary substance. Most authors, headed by Selter and Feer, decisively reject any connection with pellagra and other forms of avitaminosis. They consider F. d. an independent disease, probably of infectious origin. The infectious nature is indicated by influenza-like catarrhs at the beginning of the disease, leukocytosis, focal and island distribution of the disease. The supposed virus has a neurotropic nature: almost all symptoms can be easily explained by damage to the vegetative system, namely the vegetative centers in the intermediate brain and closely related to the vegetative system endocrine glands, first of all the chromaffin system and the thyroid gland. According to Feer, the essence of the disease comes down to a disorder of regulation of the sympathetic and parasympathetic systems. Symptoms of sympathicotonia: tachycardia, increased blood pressure, muscle hypotonia, cyanosis of hands and feet (vasospasm), hyperglycemia, increased calcium in the blood. As a result of irritation of the parasympathetic system, increased secretion from the nose, salivation and especially sweating occur. Trophic disorders are also easily explained by damage to the vegetative system. Pathological anatomy. The number of cases studied is small and the results were not always the same. Francioni and Vigi in the area of infundibuli and tuberis cinerei found degeneration of ganglion cells, and in the area of cervical sympathetic nodes infiltration in the vessel walls. Kernohan and Kennedy found changes in the basal ganglia of the brain. Whitely and Stern discovered round-cell infiltration in the spinal cord, as well as degenerative changes in the peripheral nerves. K. de Lange by serial sections found proliferation of glia both diffuse and in the form of small nodules, mainly in the tubero-infundibular area, while in the spinal cord she found nothing pathological. She, like other authors, emphasizes that the pathological changes found are insignificant compared to the severe clinical picture. Clinic. The clinical picture is quite diverse; descriptions of various authors, agreeing in the main, often differ from each other in relation to both individual symptoms and the entire course of the disease. Feer himself, who in 1923 gave a precise description of his cases, later, when his own material increased and he became aware of the observations of other authors, significantly expanded the clinical framework of the disease. According to Feer, the disease begins gradually with the above-mentioned disturbances of the general state, temperature usually remains normal; increase in temperature, if it occurs, is explained by accompanying complications (bronchitis, pyelitis, skin abscesses, etc.). Then sharply expressed sweating appears, especially on hands and feet, which at the same time become cyanotic and cold. However, according to the observations of other authors (Selter, Molchanov and others), the disease can begin acutely, with an increase in temperature to 38-39° and catarrhs of the respiratory tract. Disturbances of the general state and weakening of motor ability are expressed to varying degrees. According to Feer, in no other disease is there such a non-childish tired and depressed expression of the face; children cannot walk, stand, even sit and spend all the time in bed in a bent position. In Selter's cases, in some the disease proceeded so easily that besides poor sleep, some lethargy and typical skin changes, it gave no other phenomena, throughout the disease children remained on their feet; in other cases, convulsions, unconsciousness, severe muscle weakness were observed. Convulsions were also observed by Woringer. Tendon reflexes in rare cases are increased, more often weakened, sometimes absent. No pathological reflexes. Spinal fluid without changes, in some cases (Feer, Zechlin) Pandy and Nonne-Appelt reactions were positive. Sharply expressed and prolonged sweating is a characteristic symptom of the disease, but Swift and Zechlin in individual cases observed it only on the head. Feer himself in later reports says that sweating may not occur on hands, feet; they in such cases are dry, hot to the touch and sharply red. Besides cyanosis or redness of peripheral parts, a very common phenomenon is rash on the neck, trunk and extremities. The rash has diverse character. In some cases it is large-spotted, resembling measles, rubella or urticaria, in others-small-spotted, scarlet fever-like. The similarity with scarlet fever is further increased by the appearance of small nodules or vesicles with either clear or cloudy or purulent content (miliaria rubra, crystallina, alba) and due to abundant large-plate peeling, especially on palms and soles. Such peeling is observed even in cases where there is no sweating and rash. Therefore, Feer at present, in contrast to his previous view, together with other authors recognizes that rash and peeling do not depend on sweating, but are the result of vasomotor and trophic disorders of the skin. Trophic disorders should explain the observed in some cases loss of hair, healthy teeth, bone damage, formation of ulcers on the oral mucosa and tongue. Almost all authors point to itching of the skin as a constant phenomenon, which leads to scratching. From the side of the circulatory organs, increased pulse rate is observed-140-160, in individual cases up to 190-200 per 1 min. Blood pressure is sharply elevated: 120-140 mm Hg. Blood has been little studied. According to Feer, even in afebrile cases there is neutrophilic leukocytosis (up to 15,000). From the side of the digestive organs, besides loss of appetite and in rare cases stomatitis, as a frequent phenomenon, increased secretion of saliva is observed, as well as abdominal pains, which resemble colic.

Among other symptoms, photophobia and abundant discharge of serous fluid from the nose should be noted. Course and outcome. In most cases, the disease reaches full development within 3-4 months; regression occurs even more slowly, and the period of recovery is often interrupted by relapses. Complete recovery occurs after 4-6 months, sometimes after a year or later. Mild cases occur in which all the main symptoms do not reach full development, as well as 'attenuated forms' (formes frustes), when some of the main symptoms are absent. The outcome is not always favorable. The mortality rate is approximately 5% (zechlin, Feer). The immediate cause of death is pneumonia, sepsis. In Lange's case (child 11½ months), death occurred suddenly in the second month of the disease; at autopsy, the immediate cause of death remained unclear. - Diagnosis for a physician who is familiar with F. b. at least from the literature is not difficult in typical cases, but in the initial stages, when the cardinal symptoms are not yet expressed, as well as in 'attenuated forms', it may present considerable difficulties. The presence of one or another rash may lead to an incorrect diagnosis of scarlet fever, measles, or rubella; weakening of motor ability and muscle lethargy in the absence of tendon reflexes - to the diagnosis of polyneuritis or Heine-Medin disease; in a depressive state at the beginning of the disease, the thought of tuberculous meningitis or psychosis may arise. - Treatment, as with other infectious diseases, comes down to proper care of the child (nutrition, air, etc.). In relation to individual manifestations of the disease, treatment is symptomatic (itching, insomnia, etc.). Feer obtained excellent results from atropine (5-10 drops of a solution of Atropini sulfurici 1:1,000 during the day 2-4 times). F. b. has been insufficiently studied both in terms of clinical aspects and, especially, etiology. It is therefore not surprising that its nosological characteristic has been identified vaguely and remains indistinct; there is also no generally accepted name. Almost everyone agrees that the essence of the disease comes down to a disorder of the autonomic system. Therefore, among the proposed names, 'vegetative neurosis' should be considered the most acceptable, if neurosis is understood conditionally, allowing for the possibility of weakly expressed and non-constant organic changes in the autonomic system. Other names - acrodynia, erythredema, pink disease, dermotrophoneurosis - are much less fortunate, as they all indicate some one symptom of the disease. The name F. b. should be abandoned at least already because Feer himself renounced priority in the description of this disease; and those who necessarily want to associate the name with his name should say: 'disease Selter-Swift-Feer's', which some German pediatricians now do.